Retinoblastoma (eye cancer in children)Overview

Retinoblastoma is a rare type of eye cancer that can affect young children, usually under the age of 5.

If it's picked up early, retinoblastoma can often be successfully treated. More than 9 out of 10 children with the condition are cured.

Retinoblastoma can either affect 1 or both eyes. If it affects both eyes, it's usually diagnosed before a child is 1 year old. If it affects 1 eye, it tends to be diagnosed later (between the ages of 2 and 3).

Signs and symptoms of retinoblastoma

Signs and symptoms of retinoblastoma include:

an unusual white reflection in the pupil – this may be apparent in photos, where only the healthy eye appears red from the flash, or you may notice it in a dark or artificially lit room

a change in the colour of the iris – in 1 eye or sometimes only in 1 area of the eye

a red or inflamed eye – although your child won't usually complain of any pain

poor vision – your child may not focus on faces or objects, or they may not be able to control their eye movements (this is more common when both eyes are affected); they may say they can't see as well as they used to

These symptoms may be caused by something other than retinoblastoma. But you should get them checked by your GP as soon as possible.

It's unusual for retinoblastoma to progress unnoticed beyond the age of 5.

Signs in older children include the eye appearing red, sore or swollen, and some loss of vision in the affected eye.

What causes retinoblastoma?

Retinoblastoma is cancer of the retina. The retina is the light-sensitive lining at the back of the eye.

During the early stages of a baby's development, retinal eye cells grow very quickly and then stop growing.

But in rare cases, 1 or more cells continue to grow and form a cancer called retinoblastoma.

In about 4 out of 10 (40%) of cases, retinoblastoma is caused by a faulty gene, which often affects both eyes (bilateral).

The faulty gene may be inherited from a parent, or a change to the gene (mutation) may occur at an early stage of the child's development in the womb.

It's not known what causes the remaining 60% of retinoblastoma cases. In these cases, there's no faulty gene and only 1 eye is affected (unilateral).

Around 45 children are diagnosed with retinoblastoma in the UK each year.

Diagnosing retinoblastoma

Your GP will carry out a red reflex test in a darkened room using an ophthalmoscope (a magnifying instrument with a light at one end).

When a light is shone into your child's eyes, your GP will see a red reflection if the retina is normal.

Treating small tumours

There are 2 possible treatment options for treating small tumours contained within the eye:

laser treatment to the eye (photocoagulation or thermotherapy)

freezing the tumour (cryotherapy)

The aim of these treatments is to destroy the tumour. They're carried out under general anaesthetic, so your child will be unconscious and won't feel any pain or discomfort during the procedure.

In some cases, chemotherapy may be needed before or after these treatments.

Treating larger tumours

Larger tumours will be treated with one or a combination of the following treatments:

brachytherapy – if the tumour isn't too large, small radioactive plates called plaques are stitched over the tumour and left in place for a few days to destroy it, before being removed; radiotherapy to the whole eye may be recommended for larger tumours that haven't responded to other treatment methods

chemotherapy – may be used to shrink the tumour at the start of treatment, or it may be recommended if there's a chance of the cancer spreading; in some cases, chemotherapy medication can be delivered directly to the eye

surgery to remove the eye – is often necessary for very large tumours where there's no sight from the eye; if your child needs to have their eye removed, they'll have an artificial eye fitted in its place